The haemoglobin SS (HbSS) form of sickle cell disease is the most common and the most severe. As an inherited condition, people with HbSS receive an abnormal haemoglobin S gene from each parent.
HbSS causes substantial episodes of pain, as well as anaemia due to misshapen red blood cells. The condition can also lead to serious organ damage and other health effects if it is not properly managed.
People with sickle cell disease usually take numerous medications throughout their lives to help control the condition. Children born with the more severe form of the condition are prescribed a course of antibiotics early in life to reduce infection risk, as the condition can diminish one’s immune system response.
Certain medications can alter the shape and stickiness of abnormal red blood cells for improved blood flow. Pain medications are also common, including nonsteroidal anti-inflammatory drugs, opiates, and L-glutamine, which reduces the risk of serious complications like pain episodes and acute chest syndrome, which can severely damage the lungs.
People with HbSS disease may require repeated blood transfusions to reduce the number of abnormal red blood cells in the body. Additionally, gene therapy research is underway to determine whether it is possible to repair or replace the faulty haemoglobin gene.
Stem cell transplants (also known as marrow transplants) can effectively cure sickle cell disease, but finding a compatible donor is challenging. Transplants can also carry serious health risks of their own, which is why this treatment is often reserved for more serious forms of sickle cell disease.